CASE REPORT
Weakness in pregnancy – expect the unexpected S Furara
MRCOG*,
M Maw
MBChB MRCP†,
F Khan
MRCOG*
and K Powell
MRCOG‡
*SPR Obstetrics and Gynaecology; †SHO Medicine; ‡Consultant in Obstetrics and Gynaecology, Mid Staffordshire General Hospitals, Western Road, Stafford ST16 3SA, UK
Summary: Guillain-Barre´ syndrome (GBS) is rare in pregnancy with an incidence estimated to be between 1.2 and 1.9 cases per 100,000 people annually, and it is generally accepted that it carries a high maternal risk. Delayed diagnosis is common because the initial non-specific symptoms may mimic changes in pregnancy. GBS should be considered in any pregnant patient complaining of muscle weakness, general malaise, tingling of the fingers and respiratory discomfort. This case aims to highlight the importance of early diagnosis, allowing prompt initiation of the immunomodulatory treatments which have been shown to improve outcome alongside multidisciplinary care. Keywords: Guillain-Barre´ syndrome, acute inflammatory demyelinating polyradiculopathy, pregnancy
CASE REPORT An 18-year-old gravida 5, para 1 presented at 26 weeks of pregnancy describing pain across her shoulders and upper back, ‘pins and needles’ in her fingers and shortness of breath. She had normal reflexes and, initially, there was no demonstrable weakness. She was admitted with a provisional diagnosis of ‘hyperventilation’. It was considered that her symptoms could be psychological given the normal examination findings and blood tests. However, over the next eight days she developed an ascending weakness with speech and swallowing difficulties. She had bilateral lower motor neurone facial weakness, bulbar speech, flaccidity in the limbs, areflexia and flexor plantar responses. There was reduction in all sensory modalities in her arms and legs. A diagnosis was made of Guillain-Barre´ syndrome (GBS) and this was supported by the results of a lumbar puncture: a raised cerebrospinal fluid (CSF) protein of 1.66 g/L and a normal cell count. She had no history of preceding illnesses and serological tests for Campylobacter, Cytomegalovirus, Epstein-Barr virus (EBV) and Mycoplasma pneumoniae were negative. Antibodies to the ganglioside GQ1b were positive. On day nine she developed progressive respiratory failure and was moved to the critical care unit for regular airway assessment and subsequent ventilation. A five-day course of intravenous immunoglobulins (0.4 g/kg/day) was commenced the same day. Episodic hypertension and tachycardia, representing autonomic dysfunction, were treated with labetalol. From day 16 onwards she was gradually weaned from mechanical ventilation as her muscle power improved. Throughout her admission regular fetal monitoring and ultrasonography demonstrated a normally growing fetus. She was discharged from critical care at 29 þ 5 weeks
Correspondence to: Dr M Maw, 7 Charlotte Road, Edgbaston, Birmingham B15 2NQ, UK Email: [email protected]
gestation to a neuro-rehabilitation centre where she made an excellent recovery. There was spontaneous rupture of the membranes at 39 weeks, progressing to a normal vaginal delivery of a girl weighing 3 kg with an Apgar score of eight at one minute and nine at five minutes.
DISCUSSION GBS is an acute inflammatory demyelinating polyradiculopathy (AIDP).2 It is thought to be immune-mediated, but its pathogenesis remains uncertain.5 About two-thirds of patients have had an infection within the previous six weeks, most commonly a flu-like illness or gastroenteritis. Implicated infectious agents include M. pneumoniae, Campylobacter jejuni, Cytomegalovirus and EBV.2 The preceding infection may cause an autoimmune response with the patient’s antibodies being triggered to attack various components of the peripheral nerve myelin and sometimes the axon.5 GBS typically presents with pain, numbness, paraesthesia or weakness in the limbs and this can be mistaken for a psychological complaint,2 leading to delay in diagnosis and treatment. The interval from onset of symptoms to diagnosis in pregnancy was reported to be more than one week in 50% of cases in one review of 22 pregnant patients with GBS, attributed to initial non-specific symptoms of GBS mimicking common pregnancy complaints.1 The diagnosis of GBS depends on clinical criteria supported by CSF findings and neurophysiological testing. Essential clinical criteria are progressive motor weakness and areflexia.6 Other features include respiratory failure, facial nerve involvement, bulbar and ocular nerves (in the Miller-Fisher variant), mild sensory symptoms and autonomic dysfunction. The disease reaches its peak at one to four weeks and then, after a variable plateau phase, recovery occurs over weeks or months.2 The CSF typically shows raised protein content and a normal cell count, but it may be normal in the first week.1,6 Nerve conduction studies are abnormal in approximately 90% of cases, showing multi-focal demyelination associated with secondary axonal DOI: 10.1258/om.2008.080011. Obstetric Medicine 2008; 1: 99 –101
100
Obstetric Medicine
Volume 1
December 2008
................................................................................................................................................
Table 1
Reported cases of Guillain-Barre´ syndrome in pregnancy from 1986 to 2007
No
(Reference)
Gestation (weeks)
Specific treatment
Ventilatory support
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40
(12) (13) (14) (15) (3) (16) (16) (17) (18) (19) (19) (19) (20) (21) (21) (22) (23) (23) (24) (25) (26) (27) (28) (29) (30) (7) (7) (31) (32) (33) (34) (35) (1) (36) (37) (37) (38) (39) (40) (40)
38 14 30 40 32 18 18 21 32 26 26 32 16 35 36 36 36 35 24 29 29 10 29 34 25 7 24 6 15 21 27 4 20 36 15 15 30 16 19 13
Plasmapheresis Plasmapheresis No Plasmapheresis No No No Plasmapheresis No Plasmapheresis Plasmapheresis Plasmapheresis Plasmapheresis No Plasmapheresis Plasmapheresis Plasmapheresis No Plasmapheresis Plasmapheresis No Plasmapheresis IVIG/Plasmapheresis IVIG/Plasmapheresis IVIG IVIG/Plasmapheresis No IVIG IVIG Plasmapheresis IVIG Plasmapheresis IVIG IVIG Plasmapheresis Plasmapheresis No IVIG IVIG IVIG
No Yes Yes No No Yes Yes Yes No No No No No No No Yes No No No No No No Yes Yes No Yes No Yes Yes No No No No No No No No No Yes No
Mode of delivery CS TOP PTL Forceps CS Maternal death Forceps CS CS CS SVD CS CS CS CS CS CS SVD SVD SVD Miscarriage SVD SVD SVD CS CS TOP SVD CS SVD TOP CS SVD SVD SVD CS Unknown CS CS
Gestation at delivery 40 16 34 39 25 38 37 40 33 36 35 38 36 36 36 38 35 40 40 20 38 37 38 38 41 9 40 40 37 18 39 41 26 26
37 32
Maternal outcome
Aetiology
Died three months postpartum Died five months postpartum Died one month postpartum Recovery Recovery Rehabilitation Residual disease Recovery Recovery Recovery Recovery Residual disease Recovery Residual disease Recovery Recovery Residual disease Residual disease Recovery Recovery Recovery Recovery Recovery Recovery Recovery Recovery Recovery Recovery Recovery Recovery Recovery Recovery Residual disease Residual disease Probably recovery Probably recovery Residual disease Recovery Recovery Residual disease
Unknown CMV Unknown Unknown Unknown CMV CMV Unknown Unknown Unknown CMV CMV Unknown Unknown Unknown Unknown Unknown Unknown CMV Rubella Unknown CMV CMV Unknown Unknown Unknown EBV CMV Hepatitis A Unknown EBV Unknown Unknown Unknown CMV CMV Unknown Unknown Unknown Unknown
CMV ¼ cytomegalovirus; CS ¼ caesarean section; EBV ¼ Epstein-Barr virus; PTL ¼ preterm labour; SVD ¼ spontaneous vaginal delivery; TOP ¼ termination of pregnancy
degeneration.6 Mechanical ventilation may be required within 24 hours of symptom onset. Up to 20% of patients are disabled after one year as a result of GBS2 and a maternal mortality of 7% has been quoted (Table 1). The management of GBS in pregnancy is similar to that in the non-pregnant population7 and includes intravenous (i.v.) immunoglobulins and plasmapheresis. It is important that physicians and obstetricians manage the patient jointly.1 Ventilatory support is required in 25–30% of non-pregnant patients,2 but respiratory problems may be worse in pregnancy because of splinting of the diaphragm.8 In cases requiring ventilatory support in pregnancy, the risk of premature birth has been noted to be greatly increased.7 Thromboprophylaxis is indicated given hypercoagulability of pregnancy and immobility. Routine screening for respiratory and urinary infections is recommended. Labetalol is the agent of choice for management of autonomic dysfunction in the gravida, manifested by fluctuating pulse and blood pressure.2 This drug allows good blood pressure control without interfering with placental or fetal blood flow.9 A Cochrane review has shown that there are no outcome differences between i.v. immunoglobulins treatment and
plasmapheresis.4 In pregnancy, the safety of i.v. immunoglobulins has been established based on its use in treating conditions, such as maternal idiopathic thrombocytopenia purpura and fetal alloimmune thrombocytopenia.1 We found 12 cases of GBS in pregnancy in which i.v. immunoglobulins have been used, with no report of treatment-induced maternal or fetal complications (Table 1). In patients who have shown no signs of recovery within two weeks, a second course of i.v. immunoglobulins has shown to be beneficial.10 The benefit of plasmapheresis is great when started within seven days of disease onset, although it still provides some advantage within 30 days.5 On grounds of equivalent efficacy, similar cost, greater convenience and ease of administration, i.v. immunoglobulins have replaced plasmapheresis to be the preferred treatment in many hospitals.11 ACKNOWLEDGEMENT
We would also like to thank Dr P R Daggett (Consultant Physician, Mid Staffordshire General Hospitals) for his support in writing this case report.
Furara et al. Weakness in pregnancy
101
................................................................................................................................................
REFERENCES 1 Chan LY, Tsui MH, Leung TN. Guillain Barre´ syndrome in pregnancy. Acta Obstet Gynecol Scand 2004;83:319 –25 2 Hughes RAC, Cornblath DR. Guillain Barre´ syndrome. Lancet 2005;366:1653 –66 3 Laufenburg HF, Sirus SR. Guillain-Barre´ syndrome in pregnancy. Am Fam Phys 1989;39:147 –50 4 Hughes RA, Raphael JC, Swan AV, van Doom PA. Intravenous immunoglobulin for Guillain-Barre´ syndrome. Cochrane Database Syst Rev 2006;25:CD002063 5 Raphae¨l JC, Chevret S, Hughes RA, Annane D. Plasma exchange for Guillain-Barre´ syndrome. Cochrane Database Syst Rev. 2002;CD001798 6 Bradley WG, Daroff RB, Fenichel G, Jankovic J. Proximal, distal, and generalized weakness (chapter 28). Neurology in Clinical Practice, 4th edn. United Kingdom: Butterworth-Heinemann, 2003 7 Brooks H, Christian AS, May AE. Pregnancy, anaesthesia and Guillain Barre´ syndrome. Anaesthesia 2000;55:894– 8 8 Gautier PE, Hantson P, Vekemans MC, et al. Intensive care management of GBS during pregnancy. Inten Care Med 1990;16:460 –2 9 Lapinsky SE, Kruczynski K, Slutsky AS. Critical care in the pregnant patient. Am J Resp Crit Care Med 1995;152:427 –55 10 Farcas P, Avnun L, Frisher S, Herishana YO, Wirguin I. Efficacy of repeated intravenous immunoglobulin in severe unresponsive Guillain-Barre´ syndrome. Lancet 1997;350:1747 11 Hughes RA. Intravenous IgG in Guillain-Barre´ syndrome. Br Med J 1996;313:376 –7 12 McGrady EM. Management of labour and delivery in a patient with Guillain-Barre´ syndrome. Anaesthesia 1987;42:899 13 Hart IK, Kennedy PG. Guillain-Barre´ syndrome associated with cytomegalovirus infection. Quast J Med 1988;67:425 –30 14 Quinlan DJ, Moodley J, Lalloo BC, Nathoo UG. Guillain-Barre´ syndrome in pregnancy. A case report. S Afr Med J 1988;73:611 –2 15 Gautier PE, Pierre PA, Van Obbergh LJ, Van Steenberge A. Guillain-Barre´ syndrome after obstetrical epidural analgesia. Reg Anesth 1989;14:251 –2 16 May SE, Caudle MR, Kleinman GE. Landry-Guillain-Barre´-Strohl syndrome in pregnancy. A report of two cases. J Reprod Med 1989;34:550 –2 17 Gautier PE, Hantson P, Vekemans MC, et al. Intensive care management of Guillain-Barre´ syndrome during pregnancy. Intens. Care Med 1990;16:460– 2 18 Feldman JM. Cardiac arrest after succinylcholine administration in a pregnant patient recovered from Guillain-Barre´ syndrome. Anesthesiology 1990;72:942– 4 19 Hurley TJ, Brunson AD, Archer RL, Lefler SF, Quirk JG Jr. Landry Guillain-Barre´ Strohl syndrome in pregnancy: report of three cases treated with plasmapheresis. Obstet Gynecol 1991;78:482 –5 20 Clifton ER. Guillain-Barre´ syndrome, pregnancy, and plasmapheresis. J Am Osteopath Assoc 1992;92:1279– 82 21 Rockel A, Wissel J, Rolfs A. Guillain-Barre´ syndrome in pregnancy – an indication for caesarian section? J Perinat Med 1994;22:393– 8
22 Rolfs A, Bolik A. Guillain-Barre´ syndrome in pregnancy: reflections on immunopathogenesis. Acta Neurol Scand 1994;89:400 –2 23 Bolik A, Wissel J, Rolfs A. Guillain-Barre´ syndrome in pregnancy – two case reports and a discussion on management. Arch Gynecol Obstet 1995;256:199 –203 24 Kuller JA, Katz VL, McCoy MC, Hansen WF. Pregnancy complicated by Guillain-Barre´ syndrome. South Med J 1995;88:987 –9 25 Yaginuma Y, Kawamura M, Ishikawa M. Landry-Guillain-Barre´-Strohl syndrome in pregnancy. J Obstet Gynaecol Res 1996;22:47 –9 26 Amoah GB. Landry Guillain-Barre´-Strohl syndrome in pregnancy: case report. East Afr Med J 1996;73:623 –4 27 Mendizabal JE, Bassam BA. Guillain-Barre´ syndrome and cytomegalovirus infection during pregnancy. South Med J 1997;90:63 – 4 28 Luijckx GJ, Vles J, de Baets M, Buchwald B, Troost J. Guillain-Barre´ syndrome in mother and newborn child. Lancet 1997;349:27 29 Fait G, Gull I, Kupferminc M, et al. Intravenous immune globulins in Guillain-Barre´ syndrome in pregnancy. J Obstet Gynaecol 1998;18:78 –9 30 Seoud M, Naboulsi M, Khalil A, Sarouphim P, Azar G, Khalifeh R. Landry Guillain-Barre´ Strohl syndrome in pregnancy: use of high-dose intravenous immunoglobulin. Acta Obstet Gynecol Scand 1999;78:912 –3 31 Nesbitt I. Pregnancy, anaesthesia and Guillain-Barre´ syndrome. Anaesthesia 2000;55:1227–8 32 Breuer GS, Morali G, Finkelstein Y, Halevy J. A pregnant woman with hepatitis A and Guillain-Barre´. J Clin Gastroenterol 2001;32:179– 80 33 Vassiliev DV, Nystrom EU, Leicht CH. Combined spinal and epidural anaesthesia for labour and caesarean delivery in a patient with Guillain-Barre´ syndrome. Reg Anesth Pain Med 2001;26:174 –6 34 Yamada H, Noro N, Kato EH, Ebina Y, Cho K, Fujimoto S. Massive intravenous immunoglobulin treatment in pregnancy complicated by Guillain-Barre´ Syndrome. Eur J Obstet Gynecol Reprod Biol 2001;97:101 –4 35 Zeeman GG. A case of acute inflammatory demyelinating polyradiculoneuropathy in early pregnancy. Am J Perinatol 2001;18:213 –5 36 Wiertlewski S, Magot A, Drapier S, Malinovsky JM, Pereon Y. Worsening of neurological symptoms after epidural anaesthesia for labour in a Guillain-Barre´ patient. Anesth Analg 2004;98:825 –7 37 Magon´ T, Obrzut B, Kluz S, Chrus´ciel A, Skret A. Guillain-Barre´ syndrome during CMV infection complicating twin pregnancy – a case report. Ginekol Pol 2004;75:638 –41 38 Alici HA, Cesur M, Erdem AF, Gursac M. Repeated use of epidural anaesthesia for caesarean delivery in a patient with Guillain-Barre´ syndrome. Int J Obstet Anesth 2005;14:269– 70 39 Vijayaraghavan J, Vasudevan D, Sadique N, Rajeswari KS, Pondurangi M, Jayshree. A rare case of Guillain-Barre´ syndrome with pregnancy. J Ind Med Assoc 2006;104:269 –70 40 Vaduva C, de Seze J, Volatron AC, et al. Severe Guillain-Barre´ syndrome and pregnancy: two cases with rapid improvement post-partum. Rev Neurol (Paris) 2006;162:358 –62
(Accepted 10 June 2008)
Weakness in pregnancy – expect the unexpected S Furara
MRCOG*,
M Maw
MBChB MRCP†,
F Khan
MRCOG*
and K Powell
MRCOG‡
*SPR Obstetrics and Gynaecology; †SHO Medicine; ‡Consultant in Obstetrics and Gynaecology, Mid Staffordshire General Hospitals, Western Road, Stafford ST16 3SA, UK
Summary: Guillain-Barre´ syndrome (GBS) is rare in pregnancy with an incidence estimated to be between 1.2 and 1.9 cases per 100,000 people annually, and it is generally accepted that it carries a high maternal risk. Delayed diagnosis is common because the initial non-specific symptoms may mimic changes in pregnancy. GBS should be considered in any pregnant patient complaining of muscle weakness, general malaise, tingling of the fingers and respiratory discomfort. This case aims to highlight the importance of early diagnosis, allowing prompt initiation of the immunomodulatory treatments which have been shown to improve outcome alongside multidisciplinary care. Keywords: Guillain-Barre´ syndrome, acute inflammatory demyelinating polyradiculopathy, pregnancy
CASE REPORT An 18-year-old gravida 5, para 1 presented at 26 weeks of pregnancy describing pain across her shoulders and upper back, ‘pins and needles’ in her fingers and shortness of breath. She had normal reflexes and, initially, there was no demonstrable weakness. She was admitted with a provisional diagnosis of ‘hyperventilation’. It was considered that her symptoms could be psychological given the normal examination findings and blood tests. However, over the next eight days she developed an ascending weakness with speech and swallowing difficulties. She had bilateral lower motor neurone facial weakness, bulbar speech, flaccidity in the limbs, areflexia and flexor plantar responses. There was reduction in all sensory modalities in her arms and legs. A diagnosis was made of Guillain-Barre´ syndrome (GBS) and this was supported by the results of a lumbar puncture: a raised cerebrospinal fluid (CSF) protein of 1.66 g/L and a normal cell count. She had no history of preceding illnesses and serological tests for Campylobacter, Cytomegalovirus, Epstein-Barr virus (EBV) and Mycoplasma pneumoniae were negative. Antibodies to the ganglioside GQ1b were positive. On day nine she developed progressive respiratory failure and was moved to the critical care unit for regular airway assessment and subsequent ventilation. A five-day course of intravenous immunoglobulins (0.4 g/kg/day) was commenced the same day. Episodic hypertension and tachycardia, representing autonomic dysfunction, were treated with labetalol. From day 16 onwards she was gradually weaned from mechanical ventilation as her muscle power improved. Throughout her admission regular fetal monitoring and ultrasonography demonstrated a normally growing fetus. She was discharged from critical care at 29 þ 5 weeks
Correspondence to: Dr M Maw, 7 Charlotte Road, Edgbaston, Birmingham B15 2NQ, UK Email: [email protected]
gestation to a neuro-rehabilitation centre where she made an excellent recovery. There was spontaneous rupture of the membranes at 39 weeks, progressing to a normal vaginal delivery of a girl weighing 3 kg with an Apgar score of eight at one minute and nine at five minutes.
DISCUSSION GBS is an acute inflammatory demyelinating polyradiculopathy (AIDP).2 It is thought to be immune-mediated, but its pathogenesis remains uncertain.5 About two-thirds of patients have had an infection within the previous six weeks, most commonly a flu-like illness or gastroenteritis. Implicated infectious agents include M. pneumoniae, Campylobacter jejuni, Cytomegalovirus and EBV.2 The preceding infection may cause an autoimmune response with the patient’s antibodies being triggered to attack various components of the peripheral nerve myelin and sometimes the axon.5 GBS typically presents with pain, numbness, paraesthesia or weakness in the limbs and this can be mistaken for a psychological complaint,2 leading to delay in diagnosis and treatment. The interval from onset of symptoms to diagnosis in pregnancy was reported to be more than one week in 50% of cases in one review of 22 pregnant patients with GBS, attributed to initial non-specific symptoms of GBS mimicking common pregnancy complaints.1 The diagnosis of GBS depends on clinical criteria supported by CSF findings and neurophysiological testing. Essential clinical criteria are progressive motor weakness and areflexia.6 Other features include respiratory failure, facial nerve involvement, bulbar and ocular nerves (in the Miller-Fisher variant), mild sensory symptoms and autonomic dysfunction. The disease reaches its peak at one to four weeks and then, after a variable plateau phase, recovery occurs over weeks or months.2 The CSF typically shows raised protein content and a normal cell count, but it may be normal in the first week.1,6 Nerve conduction studies are abnormal in approximately 90% of cases, showing multi-focal demyelination associated with secondary axonal DOI: 10.1258/om.2008.080011. Obstetric Medicine 2008; 1: 99 –101
100
Obstetric Medicine
Volume 1
December 2008
................................................................................................................................................
Table 1
Reported cases of Guillain-Barre´ syndrome in pregnancy from 1986 to 2007
No
(Reference)
Gestation (weeks)
Specific treatment
Ventilatory support
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40
(12) (13) (14) (15) (3) (16) (16) (17) (18) (19) (19) (19) (20) (21) (21) (22) (23) (23) (24) (25) (26) (27) (28) (29) (30) (7) (7) (31) (32) (33) (34) (35) (1) (36) (37) (37) (38) (39) (40) (40)
38 14 30 40 32 18 18 21 32 26 26 32 16 35 36 36 36 35 24 29 29 10 29 34 25 7 24 6 15 21 27 4 20 36 15 15 30 16 19 13
Plasmapheresis Plasmapheresis No Plasmapheresis No No No Plasmapheresis No Plasmapheresis Plasmapheresis Plasmapheresis Plasmapheresis No Plasmapheresis Plasmapheresis Plasmapheresis No Plasmapheresis Plasmapheresis No Plasmapheresis IVIG/Plasmapheresis IVIG/Plasmapheresis IVIG IVIG/Plasmapheresis No IVIG IVIG Plasmapheresis IVIG Plasmapheresis IVIG IVIG Plasmapheresis Plasmapheresis No IVIG IVIG IVIG
No Yes Yes No No Yes Yes Yes No No No No No No No Yes No No No No No No Yes Yes No Yes No Yes Yes No No No No No No No No No Yes No
Mode of delivery CS TOP PTL Forceps CS Maternal death Forceps CS CS CS SVD CS CS CS CS CS CS SVD SVD SVD Miscarriage SVD SVD SVD CS CS TOP SVD CS SVD TOP CS SVD SVD SVD CS Unknown CS CS
Gestation at delivery 40 16 34 39 25 38 37 40 33 36 35 38 36 36 36 38 35 40 40 20 38 37 38 38 41 9 40 40 37 18 39 41 26 26
37 32
Maternal outcome
Aetiology
Died three months postpartum Died five months postpartum Died one month postpartum Recovery Recovery Rehabilitation Residual disease Recovery Recovery Recovery Recovery Residual disease Recovery Residual disease Recovery Recovery Residual disease Residual disease Recovery Recovery Recovery Recovery Recovery Recovery Recovery Recovery Recovery Recovery Recovery Recovery Recovery Recovery Residual disease Residual disease Probably recovery Probably recovery Residual disease Recovery Recovery Residual disease
Unknown CMV Unknown Unknown Unknown CMV CMV Unknown Unknown Unknown CMV CMV Unknown Unknown Unknown Unknown Unknown Unknown CMV Rubella Unknown CMV CMV Unknown Unknown Unknown EBV CMV Hepatitis A Unknown EBV Unknown Unknown Unknown CMV CMV Unknown Unknown Unknown Unknown
CMV ¼ cytomegalovirus; CS ¼ caesarean section; EBV ¼ Epstein-Barr virus; PTL ¼ preterm labour; SVD ¼ spontaneous vaginal delivery; TOP ¼ termination of pregnancy
degeneration.6 Mechanical ventilation may be required within 24 hours of symptom onset. Up to 20% of patients are disabled after one year as a result of GBS2 and a maternal mortality of 7% has been quoted (Table 1). The management of GBS in pregnancy is similar to that in the non-pregnant population7 and includes intravenous (i.v.) immunoglobulins and plasmapheresis. It is important that physicians and obstetricians manage the patient jointly.1 Ventilatory support is required in 25–30% of non-pregnant patients,2 but respiratory problems may be worse in pregnancy because of splinting of the diaphragm.8 In cases requiring ventilatory support in pregnancy, the risk of premature birth has been noted to be greatly increased.7 Thromboprophylaxis is indicated given hypercoagulability of pregnancy and immobility. Routine screening for respiratory and urinary infections is recommended. Labetalol is the agent of choice for management of autonomic dysfunction in the gravida, manifested by fluctuating pulse and blood pressure.2 This drug allows good blood pressure control without interfering with placental or fetal blood flow.9 A Cochrane review has shown that there are no outcome differences between i.v. immunoglobulins treatment and
plasmapheresis.4 In pregnancy, the safety of i.v. immunoglobulins has been established based on its use in treating conditions, such as maternal idiopathic thrombocytopenia purpura and fetal alloimmune thrombocytopenia.1 We found 12 cases of GBS in pregnancy in which i.v. immunoglobulins have been used, with no report of treatment-induced maternal or fetal complications (Table 1). In patients who have shown no signs of recovery within two weeks, a second course of i.v. immunoglobulins has shown to be beneficial.10 The benefit of plasmapheresis is great when started within seven days of disease onset, although it still provides some advantage within 30 days.5 On grounds of equivalent efficacy, similar cost, greater convenience and ease of administration, i.v. immunoglobulins have replaced plasmapheresis to be the preferred treatment in many hospitals.11 ACKNOWLEDGEMENT
We would also like to thank Dr P R Daggett (Consultant Physician, Mid Staffordshire General Hospitals) for his support in writing this case report.
Furara et al. Weakness in pregnancy
101
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REFERENCES 1 Chan LY, Tsui MH, Leung TN. Guillain Barre´ syndrome in pregnancy. Acta Obstet Gynecol Scand 2004;83:319 –25 2 Hughes RAC, Cornblath DR. Guillain Barre´ syndrome. Lancet 2005;366:1653 –66 3 Laufenburg HF, Sirus SR. Guillain-Barre´ syndrome in pregnancy. Am Fam Phys 1989;39:147 –50 4 Hughes RA, Raphael JC, Swan AV, van Doom PA. Intravenous immunoglobulin for Guillain-Barre´ syndrome. Cochrane Database Syst Rev 2006;25:CD002063 5 Raphae¨l JC, Chevret S, Hughes RA, Annane D. Plasma exchange for Guillain-Barre´ syndrome. Cochrane Database Syst Rev. 2002;CD001798 6 Bradley WG, Daroff RB, Fenichel G, Jankovic J. Proximal, distal, and generalized weakness (chapter 28). Neurology in Clinical Practice, 4th edn. United Kingdom: Butterworth-Heinemann, 2003 7 Brooks H, Christian AS, May AE. Pregnancy, anaesthesia and Guillain Barre´ syndrome. Anaesthesia 2000;55:894– 8 8 Gautier PE, Hantson P, Vekemans MC, et al. Intensive care management of GBS during pregnancy. Inten Care Med 1990;16:460 –2 9 Lapinsky SE, Kruczynski K, Slutsky AS. Critical care in the pregnant patient. Am J Resp Crit Care Med 1995;152:427 –55 10 Farcas P, Avnun L, Frisher S, Herishana YO, Wirguin I. Efficacy of repeated intravenous immunoglobulin in severe unresponsive Guillain-Barre´ syndrome. Lancet 1997;350:1747 11 Hughes RA. Intravenous IgG in Guillain-Barre´ syndrome. Br Med J 1996;313:376 –7 12 McGrady EM. Management of labour and delivery in a patient with Guillain-Barre´ syndrome. Anaesthesia 1987;42:899 13 Hart IK, Kennedy PG. Guillain-Barre´ syndrome associated with cytomegalovirus infection. Quast J Med 1988;67:425 –30 14 Quinlan DJ, Moodley J, Lalloo BC, Nathoo UG. Guillain-Barre´ syndrome in pregnancy. A case report. S Afr Med J 1988;73:611 –2 15 Gautier PE, Pierre PA, Van Obbergh LJ, Van Steenberge A. Guillain-Barre´ syndrome after obstetrical epidural analgesia. Reg Anesth 1989;14:251 –2 16 May SE, Caudle MR, Kleinman GE. Landry-Guillain-Barre´-Strohl syndrome in pregnancy. A report of two cases. J Reprod Med 1989;34:550 –2 17 Gautier PE, Hantson P, Vekemans MC, et al. Intensive care management of Guillain-Barre´ syndrome during pregnancy. Intens. Care Med 1990;16:460– 2 18 Feldman JM. Cardiac arrest after succinylcholine administration in a pregnant patient recovered from Guillain-Barre´ syndrome. Anesthesiology 1990;72:942– 4 19 Hurley TJ, Brunson AD, Archer RL, Lefler SF, Quirk JG Jr. Landry Guillain-Barre´ Strohl syndrome in pregnancy: report of three cases treated with plasmapheresis. Obstet Gynecol 1991;78:482 –5 20 Clifton ER. Guillain-Barre´ syndrome, pregnancy, and plasmapheresis. J Am Osteopath Assoc 1992;92:1279– 82 21 Rockel A, Wissel J, Rolfs A. Guillain-Barre´ syndrome in pregnancy – an indication for caesarian section? J Perinat Med 1994;22:393– 8
22 Rolfs A, Bolik A. Guillain-Barre´ syndrome in pregnancy: reflections on immunopathogenesis. Acta Neurol Scand 1994;89:400 –2 23 Bolik A, Wissel J, Rolfs A. Guillain-Barre´ syndrome in pregnancy – two case reports and a discussion on management. Arch Gynecol Obstet 1995;256:199 –203 24 Kuller JA, Katz VL, McCoy MC, Hansen WF. Pregnancy complicated by Guillain-Barre´ syndrome. South Med J 1995;88:987 –9 25 Yaginuma Y, Kawamura M, Ishikawa M. Landry-Guillain-Barre´-Strohl syndrome in pregnancy. J Obstet Gynaecol Res 1996;22:47 –9 26 Amoah GB. Landry Guillain-Barre´-Strohl syndrome in pregnancy: case report. East Afr Med J 1996;73:623 –4 27 Mendizabal JE, Bassam BA. Guillain-Barre´ syndrome and cytomegalovirus infection during pregnancy. South Med J 1997;90:63 – 4 28 Luijckx GJ, Vles J, de Baets M, Buchwald B, Troost J. Guillain-Barre´ syndrome in mother and newborn child. Lancet 1997;349:27 29 Fait G, Gull I, Kupferminc M, et al. Intravenous immune globulins in Guillain-Barre´ syndrome in pregnancy. J Obstet Gynaecol 1998;18:78 –9 30 Seoud M, Naboulsi M, Khalil A, Sarouphim P, Azar G, Khalifeh R. Landry Guillain-Barre´ Strohl syndrome in pregnancy: use of high-dose intravenous immunoglobulin. Acta Obstet Gynecol Scand 1999;78:912 –3 31 Nesbitt I. Pregnancy, anaesthesia and Guillain-Barre´ syndrome. Anaesthesia 2000;55:1227–8 32 Breuer GS, Morali G, Finkelstein Y, Halevy J. A pregnant woman with hepatitis A and Guillain-Barre´. J Clin Gastroenterol 2001;32:179– 80 33 Vassiliev DV, Nystrom EU, Leicht CH. Combined spinal and epidural anaesthesia for labour and caesarean delivery in a patient with Guillain-Barre´ syndrome. Reg Anesth Pain Med 2001;26:174 –6 34 Yamada H, Noro N, Kato EH, Ebina Y, Cho K, Fujimoto S. Massive intravenous immunoglobulin treatment in pregnancy complicated by Guillain-Barre´ Syndrome. Eur J Obstet Gynecol Reprod Biol 2001;97:101 –4 35 Zeeman GG. A case of acute inflammatory demyelinating polyradiculoneuropathy in early pregnancy. Am J Perinatol 2001;18:213 –5 36 Wiertlewski S, Magot A, Drapier S, Malinovsky JM, Pereon Y. Worsening of neurological symptoms after epidural anaesthesia for labour in a Guillain-Barre´ patient. Anesth Analg 2004;98:825 –7 37 Magon´ T, Obrzut B, Kluz S, Chrus´ciel A, Skret A. Guillain-Barre´ syndrome during CMV infection complicating twin pregnancy – a case report. Ginekol Pol 2004;75:638 –41 38 Alici HA, Cesur M, Erdem AF, Gursac M. Repeated use of epidural anaesthesia for caesarean delivery in a patient with Guillain-Barre´ syndrome. Int J Obstet Anesth 2005;14:269– 70 39 Vijayaraghavan J, Vasudevan D, Sadique N, Rajeswari KS, Pondurangi M, Jayshree. A rare case of Guillain-Barre´ syndrome with pregnancy. J Ind Med Assoc 2006;104:269 –70 40 Vaduva C, de Seze J, Volatron AC, et al. Severe Guillain-Barre´ syndrome and pregnancy: two cases with rapid improvement post-partum. Rev Neurol (Paris) 2006;162:358 –62
(Accepted 10 June 2008)
